Hirayama Disease: Case Report.

A 26-year-old previously healthy patient who, at the age of 18 years, began progressive loss of distal strength, rest tremor, and muscle atrophy in the left upper limb. Upon examination, the patient presented moderate distal atrophy, degree 4 in muscular strength, and minipolymioclonus. Electromyoneurography revealed (EMNG) chronic preganglionic bilateral involvement of bilateral C7/C8/T1, worse on the left, with signs of active C8/T1 denervation. A cervical spine magnetic resonance imaging (MRI) scan showed spondylodiscal degenerative changes with central protrusions in C4-C5, C6-C7, and right central in C5-C6, which touched the dural sac. The anteroposterior diameter of the medulla in neutral position, in the C5-C6 plane, was of 5.1 mm. There was a reduction of the spinal cord caliber to 4.0 mm after the dynamic maneuver of forced flexion of the spine, as well as signal increase in the anterior horns. The clinical findings and those of the complementary tests were compatible with Hirayama disease (HD), a rare benign motor neuron disease that affects cervical spinal segments and is most prevalent in men, with onset in the early 20s. Unilateral and slowly progressive weakness is typical, but self-limited. Sensory disturbances, and autonomic and upper motor neuron signals are rare. Management is usually conservative, with the use of a soft cervical collar. Although rare, HD should be considered in young patients with focal asymmetric atrophy in the upper limbs. The early diagnosis of HD depends on the degree of suspicion, as well as on the cooperation and communication among the various specialties involved in the investigation.

Hirayama Disease: Case Report / Doença de Hirayama: Relato de caso

Abstract A 26-year-old previously healthy patient who, at the age of 18 years, began progressive loss of distal strength, rest tremor, and muscle atrophy in the left upper limb. Upon examination, the patient presented moderate distal atrophy, degree 4 in muscular strength, and minipolymioclonus. Electromyoneurography revealed (EMNG) chronic preganglionic bilateral involvement of bilateral C7/C8/T1, worse on the left, with signs of active C8/T1 denervation. A cervical spine magnetic resonance imaging (MRI) scan showed spondylodiscal degenerative changes with central protrusions in C4-C5, C6-C7, and right central in C5-C6, which touched the dural sac. The anteroposterior diameter of the medulla in neutral position, in the C5-C6 plane, was of 5.1 mm. There was a reduction of the spinal cord caliber to 4.0 mm after the dynamic maneuver of forced flexion of the spine, as well as signal increase in the anterior horns. The clinical findings and those of the complementary tests were compatible with Hirayama disease (HD), a rare benign motor neuron disease that affects cervical spinal segments and is most prevalent in men, with onset in the early 20s. Unilateral and slowly progressive weakness is typical, but self-limited. Sensory disturbances, and autonomic and upper motor neuron signals are rare. Management is usually conservative, with the use of a soft cervical collar. Although rare, HD should be considered in young patients with focal asymmetric atrophy in the upper limbs. The early diagnosis of HD depends on the degree of suspicion, as well as on the cooperation and communication among the various specialties involved in the investigation.

Resumo Paciente de 26 anos, previamente hígido, que, aos 18 anos, iniciou perda progressiva de força distal, tremor de repouso, e atrofia muscular no membro superior esquerdo. Ao exame, apresentou atrofia moderada, distal, força muscular de grau 4, e minipolimioclonus. A eletroneuromiografia (ENMG) revelou comprometimento pré-ganglionar crônico de C7/C8/T1 bilateral pior à esquerda, com sinais de desnervação ativa em C8/T1. A ressonância magnética (RM) de coluna cervical mostrou alterações degenerativas espondilodiscais com protrusões centrais em C4-C5, C6-C7, e central direita em C5-C6, que tocavam o saco dural. O diâmetro anteroposterior da medula na posição neutra, no plano de C5-C6, era de 5,1 mm. Houve redução do calibre da medula para 4,0 mm após a manobra dinâmica de flexão forçada da coluna, e aumento de sinal nos cornos anteriores. Os achados clínicos e os dos exames complementares eram compatíveis com doença de Hirayama (DH), uma doença benigna rara dos neurônios motores, que afeta os segmentos espinhais cervicais e é mais prevalente em homens e de início próximo aos 20 anos. É típica a fraqueza unilateral e lentamente progressiva, porém autolimitada. Perturbações sensoriais, sinais autonômicos e do neurônio motor superior são raras. O manejo geralmente é conservador, com uso de colar cervical macio. Apesar de rara, a DH deve ser considerada em pacientes jovens que apresentam atrofias assimétricas focais de membros superiores. O diagnóstico precoce de DH depende do grau de suspeição, e da cooperação e comunicação entre as diversas especialidades envolvidas na investigação.

Evaluation of intravascular contrast media transit times in coronary computed tomography angiography.

Objective: To measure the transit times (TTs) of contrast agents among the injection site (antecubital vein), superior vena cava, pulmonary trunk, and ascending aorta, in coronary computed tomography angiography (CTA) examinations of outpatients with no history of cardiovascular or lung disease, thus defining reference values for those TTs. Materials and Methods: The contrast TTs from the injection site (antecubital vein) to the superior vena cava, from the superior vena cava to the pulmonary trunk, and from the pulmonary trunk to the ascending aorta were measured by monitoring contrast enhancement in real time (bolus tracking). Cardiac output was measured by the geometric method during the CTA examination and was correlated with the contrast TT. Results: Forty-three individuals were analyzed. The mean contrast TT was 13.1 s overall (from the antecubital vein to the ascending aorta), 3.0 s from the superior vena cava to the pulmonary trunk, and 7.2 s from the pulmonary trunk to the ascending aorta. There was a tendency toward a correlation between contrast TT and cardiac output (p = 0.055). Conclusion: The reference values established here for contrast TTs among the superior vena cava, pulmonary trunk, and ascending aorta will serve as a basis for clinical evaluation.

Objetivo: Mensurar os tempos de trânsito de contraste (TTCs) entre o sítio de injeção em veia antecubital e a veia cava superior, tronco arterial pulmonar e aorta ascendente em exames de tomografia computadorizada de artérias coronárias de pacientes sem história de doenças cardiovasculares ou pulmonares, definindo padrões de normalidade para esses tempos de circulação. Materiais e Métodos: Os TTCs entre o sítio de injeção e a veia cava superior, tronco arterial pulmonar e aorta ascendente foram medidos com base nas imagens de monitoração (bolus tracking). O débito cardíaco foi calculado com base nas imagens de angiotomografia computadorizada pelo método geométrico e correlacionado com os TTCs. Resultados: Foram analisados 43 pacientes. O TTC médio entre o tronco arterial pulmonar e a aorta ascendente foi de 7,2 s, entre a veia cava superior e o tronco arterial pulmonar foi de 3 s e entre a veia antecubital e a aorta ascendente foi de 13 s. Houve tendência a correlação entre o TTC e o débito cardíaco, com valor de p de 0,055. Conclusão: Os valores de normalidade do TTC entre a veia cava superior, tronco arterial pulmonar e aorta ascendente foram estabelecidos, servindo de base para avaliação clínica.

Avaliação de diferentes tempos de trânsito do meio de contraste intravascular em exames de tomografia computadorizada coronariana / Evaluation of intravascular contrast media transit times in coronary computed tomography angiography

Resumo Objetivo: Mensurar os tempos de trânsito de contraste (TTCs) entre o sítio de injeção em veia antecubital e a veia cava superior, tronco arterial pulmonar e aorta ascendente em exames de tomografia computadorizada de artérias coronárias de pacientes sem história de doenças cardiovasculares ou pulmonares, definindo padrões de normalidade para esses tempos de circulação. Materiais e Métodos: Os TTCs entre o sítio de injeção e a veia cava superior, tronco arterial pulmonar e aorta ascendente foram medidos com base nas imagens de monitoração (bolus tracking). O débito cardíaco foi calculado com base nas imagens de angiotomografia computadorizada pelo método geométrico e correlacionado com os TTCs. Resultados: Foram analisados 43 pacientes. O TTC médio entre o tronco arterial pulmonar e a aorta ascendente foi de 7,2 s, entre a veia cava superior e o tronco arterial pulmonar foi de 3 s e entre a veia antecubital e a aorta ascendente foi de 13 s. Houve tendência a correlação entre o TTC e o débito cardíaco, com valor de p de 0,055. Conclusão: Os valores de normalidade do TTC entre a veia cava superior, tronco arterial pulmonar e aorta ascendente foram estabelecidos, servindo de base para avaliação clínica.

Abstract Objective: To measure the transit times (TTs) of contrast agents among the injection site (antecubital vein), superior vena cava, pulmonary trunk, and ascending aorta, in coronary computed tomography angiography (CTA) examinations of outpatients with no history of cardiovascular or lung disease, thus defining reference values for those TTs. Materials and Methods: The contrast TTs from the injection site (antecubital vein) to the superior vena cava, from the superior vena cava to the pulmonary trunk, and from the pulmonary trunk to the ascending aorta were measured by monitoring contrast enhancement in real time (bolus tracking). Cardiac output was measured by the geometric method during the CTA examination and was correlated with the contrast TT. Results: Forty-three individuals were analyzed. The mean contrast TT was 13.1 s overall (from the antecubital vein to the ascending aorta), 3.0 s from the superior vena cava to the pulmonary trunk, and 7.2 s from the pulmonary trunk to the ascending aorta. There was a tendency toward a correlation between contrast TT and cardiac output (p = 0.055). Conclusion: The reference values established here for contrast TTs among the superior vena cava, pulmonary trunk, and ascending aorta will serve as a basis for clinical evaluation.

Non-coding deletions identify Maenli lncRNA as a limb-specific En1 regulator.

Long non-coding RNAs (lncRNAs) can be important components in gene-regulatory networks1, but the exact nature and extent of their involvement in human Mendelian disease is largely unknown. Here we show that genetic ablation of a lncRNA locus on human chromosome 2 causes a severe congenital limb malformation. We identified homozygous 27-63-kilobase deletions located 300 kilobases upstream of the engrailed-1 gene (EN1) in patients with a complex limb malformation featuring mesomelic shortening, syndactyly and ventral nails (dorsal dimelia). Re-engineering of the human deletions in mice resulted in a complete loss of En1 expression in the limb and a double dorsal-limb phenotype that recapitulates the human disease phenotype. Genome-wide transcriptome analysis in the developing mouse limb revealed a four-exon-long non-coding transcript within the deleted region, which we named Maenli. Functional dissection of the Maenli locus showed that its transcriptional activity is required for limb-specific En1 activation in cis, thereby fine-tuning the gene-regulatory networks controlling dorso-ventral polarity in the developing limb bud. Its loss results in the En1-related dorsal ventral limb phenotype, a subset of the full En1-associated phenotype. Our findings demonstrate that mutations involving lncRNA loci can result in human Mendelian disease.

Heidenhain variant of Creutzfeldt-Jakob disease in Brazil: a case report / Variante Heidenhain da Doença de Creutzfeldt-Jakob no Brasil: um relato de caso

Creutzfeldt-Jakob disease (CJD) is a rare spongiform encephalopathy characterized by a rapid neurodegenerative progress, caused by a misfolded variant of the cellular prion protein (PrP) known as PrPSc. The clinical presentation of sCJD includes a wide range of neurological signs of cortical, subcortical, or cerebellar origin, either isolated or in various combinations. Due to this protean clinical presentation form, sCJD must be distinguished from other dementias. In this case report, we discuss the Heidenhain variant of Creutzfeldt-Jakob disease (HvCJD), a rare variant characterized by early visual symptoms and typical findings in imaging scans. Our patient presented rapidly progressive dementia and a history of visual hallucinations. As for other prion diseases, only symptomatic treatment is available for HvCJD. Thirty years of clinical investigation of patients with prion disease have resulted in little progress in either defining or evaluating potential treatments.

A doença de Creutzfeldt-Jakob (DCJ) é uma encefalopatia rara caracterizada por rápida progressão neurodegenerativa, causada pelo enovelamento incorreto da proteína priônica celular (PrP), conhecido como PrPSc. O quadro clínico da DCJ esporádica inclui um amplo espectro de sinais neurológicos de origens cortical, subcortical ou cerebelar, seja de forma isolada, seja combinada. Por causa da sua apresentação clínica variável, a DCJ esporádica deve ser distinguida de outras demências. Neste relato de caso, discutimos a variante Heidenhain da DCJ (vHDCJ), uma variante rara caracterizada por sintomas visuais precoces e características específicas no exame de imagem. Nossa paciente apresentou demência rapidamente progressiva e histórico de alucinações visuais. Assim como para as demais doenças priônicas, apenas o tratamento sintomático está disponível para a vHDCJ. Trinta anos de investigação clínica de pacientes com doença priônica têm resultado em pouco progresso, seja definindo os potenciais tratamentos, seja avaliando-os.

Contrast-Enhanced Mammography (CEM) for Detecting Residual Disease after Neoadjuvant Chemotherapy: A Comparison with Breast Magnetic Resonance Imaging (MRI).

OBJECTIVE: To evaluate the performance of contrast-enhanced mammography (CEM) compared to magnetic resonance imaging (MRI) for estimating residual tumor size after neoadjuvant chemotherapy (NAC) in women with newly diagnosed breast cancer. METHODS: The institutional review board approved this study. This prospective study included women with newly diagnosed breast cancer who underwent breast CEM and MRI at the end of the last cycle of NAC and before definitive surgery. Size of residual malignancy on post-NAC CEM and MRI was compared with surgical pathology. Agreements and correlations of CEM and MRI measurements with histological size were assessed. RESULTS: Thirty-three patients were included with a mean age of 45 years (range 22-76). The sensitivity, specificity, and positive and negative predictive value for detection of residual disease of CEM were 76%, 87.5%, 95%, and 86.4%, and those of MRI were 92%, 75%, 92%, and 75%. Comparing CEM to MRI, the mean difference was -0.8 cm, concordance coefficient was 0.7, and Pearson correlation was 0.7 (p = 0.0003). The concordance coefficient between measurements of each imaging modality and pathologic tumor size was 0.7 for CEM and 0.4 for MRI. Pearson correlation was 0.8 for CEM and 0.5 for MRI. Mean differences between CEM, MRI, and residual histopathological tumor size were 0.8 cm and 1.8 cm, respectively. CONCLUSIONS: CEM has good correlation and agreement with histopathology for measuring residual disease after NAC. CEM was comparable to MRI, showing high positive predictive value and specificity for detecting residual disease.

Pregnancy-induced growth of a spinal hemangioblastoma: presumed mechanisms and their implications for therapeutic approaches.

Hemangioblastomas are benign tumors of the central nervous system (CNS) that may occur either sporadically or as part of von Hippel-Lindau (VHL) disease, in which they coexist with a series of other tumors outside the CNS. Because of their low mitosis rate, hemangioblastomas usually have slow-growing and late manifestations, but may cause sudden neurological symptoms if tumor hemorrhage occurs. Few studies have evaluated the impact of pregnancy on the evolution of hemangioblastomas. Some authors have reported tumor growth in women with VHL disease, but no such association was observed by others. The influence of pregnancy on sporadic hemangioblastomas remains largely unexplored. We report here the case of a pregnant woman whose first manifestation of sporadic spinal hemangioblastoma was life-threatening, rapidly progressive dysautonomia. In addition, we discuss the role of pregnancy in the triggering of symptoms, as well as the possibility of medically indicated delivery for the management of these tumors.

Accuracy of contrast-enhanced spectral mammography for estimating residual tumor size after neoadjuvant chemotherapy in patients with breast cancer: a feasibility study.

OBJECTIVE: To assess the feasibility of contrast-enhanced spectral mammography (CESM) of the breast for assessing the size of residual tumors after neoadjuvant chemotherapy (NAC). MATERIALS AND METHODS: In breast cancer patients who underwent NAC between 2011 and 2013, we evaluated residual tumor measurements obtained with CESM and full-field digital mammography (FFDM). We determined the concordance between the methods, as well as their level of agreement with the pathology. Three radiologists analyzed eight CESM and FFDM measurements separately, considering the size of the residual tumor at its largest diameter and correlating it with that determined in the pathological analysis. Interobserver agreement was also evaluated. RESULTS: The sensitivity, specificity, positive predictive value, and negative predictive value were higher for CESM than for FFDM (83.33%, 100%, 100%, and 66% vs. 50%, 50%, 50%, and 25%, respectively). The CESM measurements showed a strong, consistent correlation with the pathological findings (correlation coefficient = 0.76-0.92; intraclass correlation coefficient = 0.692-0.886). The correlation between the FFDM measurements and the pathological findings was not statistically significant, with questionable consistency (intraclass correlation coefficient = 0.488-0.598). Agreement with the pathological findings was narrower for CESM measurements than for FFDM measurements. Interobserver agreement was higher for CESM than for FFDM (0.94 vs. 0.88). CONCLUSION: CESM is a feasible means of evaluating residual tumor size after NAC, showing a good correlation and good agreement with pathological findings. For CESM measurements, the interobserver agreement was excellent.

OBJETIVO: Avaliar a viabilidade da utilização da mamografia espectral com meio de contraste (CESM) na avaliação do tumor residual em mulheres com câncer de mama submetidas a quimioterapia neoadjuvante. MATERIAIS E MÉTODOS: Foi avaliada a concordância entre a mensuração do tumor residual na CESM e na mamografia digital (FFDM) com os dados histopatológicos de mulheres submetidas a quimioterapia neoadjuvante entre 2011 e 2013. Após as exclusões, três radiologistas analisaram oito CESMs e FFDMs separadamente. A maior dimensão do tumor residual foi considerada para comparação com os resultados histopatológicos. Concordância e correlação da CESM e FFDM com resultados histopatológicos e a concordância interobservador foram avaliadas. RESULTADOS: A CESM teve sensibilidade, especificidade e valores preditivos positivos e negativos maiores que a FFDM - 83,33%, 100%, 100% e 66% versus 50%, 50%, 50% e 25%, respectivamente. A CESM teve correlação boa e consistente com os achados histopatológicos (coeficiente de correlação = 0,76-0,92; coeficiente de correlação intraclasse = 0,692-0,886). A correlação entre FFDM e os achados histopatológicos não foi estatisticamente significante, com consistência questionável (coeficiente de correlação intraclasse = 0,488-0,598). A concordância entre as dimensões do estudo histopatológico foi mais estreita com a CESM do que com a FFDM. A concordância interobservador foi maior na CESM (0,94) do que na FFDM (0,88). CONCLUSÃO: A CESM é viável e pode ser utilizada para avaliação de tumor residual após quimioterapia neoadjuvante. A CESM tem boa correlação e concordância com o estudo histopatológico e excelente concordância interobservador.

Accuracy of contrast-enhanced spectral mammography for estimating residual tumor size after neoadjuvant chemotherapy in patients with breast cancer: a feasibility study / Acurácia da mamografia espectral com contraste para seguimento de tumor residual pós-quimioterapia neoadjuvante em pacientes com câncer de mama: um estudo de viabilidade

Abstract Objective: To assess the feasibility of contrast-enhanced spectral mammography (CESM) of the breast for assessing the size of residual tumors after neoadjuvant chemotherapy (NAC). Materials and methods: In breast cancer patients who underwent NAC between 2011 and 2013, we evaluated residual tumor measurements obtained with CESM and full-field digital mammography (FFDM). We determined the concordance between the methods, as well as their level of agreement with the pathology. Three radiologists analyzed eight CESM and FFDM measurements separately, considering the size of the residual tumor at its largest diameter and correlating it with that determined in the pathological analysis. Interobserver agreement was also evaluated. Results: The sensitivity, specificity, positive predictive value, and negative predictive value were higher for CESM than for FFDM (83.33%, 100%, 100%, and 66% vs. 50%, 50%, 50%, and 25%, respectively). The CESM measurements showed a strong, consistent correlation with the pathological findings (correlation coefficient = 0.76-0.92; intraclass correlation coefficient = 0.692-0.886). The correlation between the FFDM measurements and the pathological findings was not statistically significant, with questionable consistency (intraclass correlation coefficient = 0.488-0.598). Agreement with the pathological findings was narrower for CESM measurements than for FFDM measurements. Interobserver agreement was higher for CESM than for FFDM (0.94 vs. 0.88). Conclusion: CESM is a feasible means of evaluating residual tumor size after NAC, showing a good correlation and good agreement with pathological findings. For CESM measurements, the interobserver agreement was excellent.

Resumo Objetivo: Avaliar a viabilidade da utilização da mamografia espectral com meio de contraste (CESM) na avaliação do tumor residual em mulheres com câncer de mama submetidas a quimioterapia neoadjuvante. Materiais e métodos: Foi avaliada a concordância entre a mensuração do tumor residual na CESM e na mamografia digital (FFDM) com os dados histopatológicos de mulheres submetidas a quimioterapia neoadjuvante entre 2011 e 2013. Após as exclusões, três radiologistas analisaram oito CESMs e FFDMs separadamente. A maior dimensão do tumor residual foi considerada para comparação com os resultados histopatológicos. Concordância e correlação da CESM e FFDM com resultados histopatológicos e a concordância interobservador foram avaliadas. Resultados: A CESM teve sensibilidade, especificidade e valores preditivos positivos e negativos maiores que a FFDM - 83,33%, 100%, 100% e 66% versus 50%, 50%, 50% e 25%, respectivamente. A CESM teve correlação boa e consistente com os achados histopatológicos (coeficiente de correlação = 0,76-0,92; coeficiente de correlação intraclasse = 0,692-0,886). A correlação entre FFDM e os achados histopatológicos não foi estatisticamente significante, com consistência questionável (coeficiente de correlação intraclasse = 0,488-0,598). A concordância entre as dimensões do estudo histopatológico foi mais estreita com a CESM do que com a FFDM. A concordância interobservador foi maior na CESM (0,94) do que na FFDM (0,88). Conclusão: A CESM é viável e pode ser utilizada para avaliação de tumor residual após quimioterapia neoadjuvante. A CESM tem boa correlação e concordância com o estudo histopatológico e excelente concordância interobservador.

Correlation of clinical findings and brain volume data in multiple sclerosis.

Brain volume measurements are becoming an important tool for assessing success in controlling multiple sclerosis (MS) activity. MSmetrix (icometrix) is an easy-to-use platform, specific for MS magnetic resonance imaging (MRI) of the brain. It provides data on total brain volume, grey matter volume and lesion load volume. The objective of the present study was to assess whether disability and the number of relapses during the previous year correlated with brain volume measurements from MSmetrix. Data on 185 icometrix reports from patients with MS were used to evaluate the potential correlation between brain volume measurements and clinical parameters. There was a significant correlation between higher disability and decreased brain volume (total and grey matter). Increased lesion load in the brain and higher number of relapses in the previous year were also independently correlated with decreased brain tissue volume and with increased disability. This is the first study with real-world data to show that icometrix is a relevant tool for the study of brain volume loss in MS.

Angiossarcoma primário de mama: desa o diagnóstico / Primary breast angiosarcoma: a diagnostic challenge

Os autores expõem um caso de uma mulher de 57 anos que apresentou uma massa palpável na mama direita. Na mamogra a, foi identi cada uma assimetria focal com gordura de permeio, sem achado suspeito à ultrassonogra a. A ressonância magnética das mamas, com meio de con­ traste, demonstrou um nódulo irregular e mal de nido, com baixo sinal em T1 sem supressão de gordura e alto sinal em T2 com supressão de gordura. Apresentou também realce heterogêneo irregular com septos espessos realçantes medindo 5,3 cm, cinética inicial rápida e curva do tipo persistente. Uma nova ultrassonogra a dirigida foi realizada, sendo observado um nódulo hi­ poecoico de 1,2 cm, associado à alteração da ecogenicidade do parênquima mamário adjacente. Realizou­se biópsia a vácuo do nódulo hipoecoico e do parênquima adjacente, guiada por ultras­ som. O estudo histopatológico demonstrou angiossarcoma primário da mama de baixo grau. O PET­CT, realizado 6 meses após a cirurgia, não evidenciou recidiva ou metástases.

The authors display a case of a woman aged 57 years who presented a palpable mass in the right breast. The mammography revelled a focal asymmetry, with no suspicious ndings on ultrasound. Breast MRI showed an irregular and unde ned mass with low signal intensity on T1 without fat suppression and high signal on T2­weighted fat­suppressed, with irregular heterogeneous enhancement with thick enhancement septa, fast initial kinetic enhancement and persistent type curve, measuring 5.3 cm. A new ultrasound exam was performed, observing hypoechoic nodule of 1.2 cm associated with changes in echogenicity of the adjacent breast parenchyma. Ultrasound­guided vacuum biopsy of the hypoechoic nodule and the adjacent parenchyma was performed. Histopathological study showed primary breast angiosarcoma of low­grade. The PET­CT performed six months after surgery showed no recurrence or metastasis.

An 81-year-old male with iliopsoas abscess by Streptococcus sanguis.

Iliopsoas muscle abscess is an uncommon condition, which has been growing in incidence. We describe a primary iliopsoas abscess by Streptococcus sanguis affecting an 81-year-old man cured by antibiotic therapy and aspiration procedure. The objective is to enhance the suspicion index about the iliopsoas abscess that may be mistaken for other causes of acute abdomen. The important diagnostic role of abdominal imaging studies is also emphasised.

Stroke: bowel dysfunction in patients admitted for rehabilitation.

AIM: to assess the prevalence of diminished frequency of bowel movements, lumpy or hard stools, intestinal constipation, straining, incomplete evacuation, incontinence (bowel dysfunctions) in patients with brain injury resulting from cerebrovascular accident, either self-reported or reported by their caregivers; to describe the type and frequency of such dysfunctions; and the prevalence of laxative use both before and after stroke. METHOD: cross-sectional study with 98 hospitalized patients admitted for rehabilitation between December 2009 and May 2010. RESULTS: the prevalence of bowel dysfunctions before stroke was 23.96% whereas after the lesion it was 55.21% (p<0.0001). As reported by patients/caregivers, the chances of developing bowel dysfunctions increase sevenfold after stroke, 95% CI (2.44-24.26). The most frequent dysfunctions before stroke were intestinal constipation (73.91%) and diminished frequency of bowel movements (17.39%). After stroke, constipation remains to be the most frequent dysfunction reported (50%), followed by diminished frequency of bowel movements (26.79%), incomplete evacuation (12.50%), and lack of privacy (5.36%). The use of laxatives was 19,15% after the lesion, but not statisticaly significant (p=0.0736). CONCLUSION: Bowel dysfunctions increases significantly after stroke. Therefore, further studies are needed to better understand and characterize such dysfunctions, which are scarcely described in the literature.

Update on middle ear barotrauma after hyperbaric oxygen therapy - Insights on pathophysiology

Introduction: Middle ear barotrauma is the most common side effect of hyperbaric oxygen therapy. Knowledge and understanding of its pathophysiology are crucial for an accurate diagnosis and proper decision making about treatment and prevention. Objective: Describe up-to-date information on pathophysiology of middle ear barotrauma after hyperbaric oxygen therapy considering the physiology of pressure variation of the middle ear. Data Synthesis Middle ear barotrauma occurs especially during the compression phase of hyperbaric oxygen therapy. The hyperoxic environment in hyperbaric oxygen therapy leads to ventilatory dysfunction of the eustachian tube, especially in monoplace chambers, where the patients are pressurized with 100% O2, favoring middle ear barotrauma. Conclusion: The eustachian tube, the tympanic cavity, and mastoid work together in a neural controlled feedback system in which various mechanisms concur for middle ear pressure regulation...

Brain MRI and magnetic resonance spectroscopy findings in patients with hyperargininemia.

BACKGROUND AND PURPOSE: Hyperargininemia (HA) is a rare autosomal recessive metabolic disorder and the neuroimaging features of this disease have seldom been reported. Hyperammonemic encephalopathy is uncommon in HA, and the clinical presentation of HA is distinct from other urea cycle disorders. This paper describes the brain MRI findings and a magnetic resonance spectroscopy (MRS) study of a series of Brazilian HA patients. METHODS: Brain MR images were obtained in eight male and two female patients with the classic HA phenotype. Six patients were evaluated twice. Single-voxel (1)H-MRS was also performed in six of the patients. RESULTS: Only 1 patient, with less severe neurological symptoms, had normal MRI images. A variable degree of cerebral atrophy was noted in the other patients, and 3 patients also presented mild symptoms of cerebellar atrophy. MRS indicated no metabolic abnormalities in any patient. CONCLUSIONS: We present the MRI and MRS findings of a large series of HA patients. Variable degrees of brain atrophy and mild cerebellar atrophy were observed, and these findings were not specific. No metabolic abnormality was observed using MRS in this series of patients.

Update on middle ear barotrauma after hyperbaric oxygen therapy-insights on pathophysiology.

Introduction Middle ear barotrauma is the most common side effect of hyperbaric oxygen therapy. Knowledge and understanding of its pathophysiology are crucial for an accurate diagnosis and proper decision making about treatment and prevention. Objective Describe up-to-date information on pathophysiology of middle ear barotrauma after hyperbaric oxygen therapy considering the physiology of pressure variation of the middle ear. Data Synthesis Middle ear barotrauma occurs especially during the compression phase of hyperbaric oxygen therapy. The hyperoxic environment in hyperbaric oxygen therapy leads to ventilatory dysfunction of the eustachian tube, especially in monoplace chambers, where the patients are pressurized with 100% O2, favoring middle ear barotrauma. Conclusion The eustachian tube, the tympanic cavity, and mastoid work together in a neural controlled feedback system in which various mechanisms concur for middle ear pressure regulation.

Mastoid surface area-to-volume ratios in adult Brazilian individuals.

UNLABELLED: Computed tomography-based measurements of mastoid surface area and volume have not been described for adult Brazilian individuals. These measurements add to the understanding of middle ear physiology, related diseases, and their impact on mastoid pneumatization. OBJECTIVE: To check the ratio between mastoid surface area and volume. METHOD: This contemporary cross-sectional cohort study included 28 patients submitted to CT imaging of the mastoid. Measurements of surface area and volume were made based on serial CT scans and produced with the aid of software package Image Pro Plus. RESULTS: Mastoid volumes ranged from 5.5 to 72.4 cm³. Surface areas ranged from 43.9 to 525.2 cm². Surface areas varied linearly with volumes. CONCLUSION: Mastoid surface areas and volumes of adult Brazilian subjects followed a linear correlation, as also described in studies carried out in other countries. Mean surface areas and volumes were higher than previously published, unlike surface area-to-volume ratios, which were lower. Further studies with larger populations will provide evidence as to whether Brazilian subjects have larger surface areas and volumes than other populations.

Relação entre área e volume da mastoide em adultos brasileiros / Mastoid surface area-to-volume ratios in adult Brazilian individuals

As medidas de área e volume da mastoide por tomografia computadorizada não foram previamente descritas em adultos brasileiros. O conhecimento dessas medidas é muito importante para o entendimento da fisiologia da orelha média e seus estados patológicos, refletindo diretamente na pneumatização mastoídea. OBJETIVO: Verificar a existência de relação entre a área e o volume da mastoide. MÉTODO: Estudo de coorte contemporânea com corte transversal, no qual 28 pacientes foram submetidos à tomografia de mastoides. Foram reconstruídas as medidas de área e volume das mastoides por cortes seriados usando o software Image Pro Plus. RESULTADOS: Os volumes das mastoides variaram de 5,5 até 72,4 cm³. As áreas variaram de 43,9 até 525,2 cm². A área da mastoide foi uma função linear do volume. CONCLUSÃO: As medidas de área e volume da mastoide em adultos brasileiros seguem uma correlação linear, concordando com estudos realizados em outros países. As médias de área e volume encontradas foram superiores às previamente publicadas, ao contrário da média da relação área/volume, que foi inferior. Futuros estudos, com número maior de indivíduos, poderão esclarecer se a população brasileira tem ou não maiores médias de área e volume da mastoide em relação a outras populações específicas.

Computed tomography-based measurements of mastoid surface area and volume have not been described for adult Brazilian individuals. These measurements add to the understanding of middle ear physiology, related diseases, and their impact on mastoid pneumatization. OBJECTIVE: To check the ratio between mastoid surface area and volume. METHOD: This contemporary cross-sectional cohort study included 28 patients submitted to CT imaging of the mastoid. Measurements of surface area and volume were made based on serial CT scans and produced with the aid of software package Image Pro Plus. RESULTS: Mastoid volumes ranged from 5.5 to 72.4 cm³. Surface areas ranged from 43.9 to 525.2 cm². Surface areas varied linearly with volumes. CONCLUSION: Mastoid surface areas and volumes of adult Brazilian subjects followed a linear correlation, as also described in studies carried out in other countries. Mean surface areas and volumes were higher than previously published, unlike surface area-to-volume ratios, which were lower. Further studies with larger populations will provide evidence as to whether Brazilian subjects have larger surface areas and volumes than other populations.